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Journal of Clinical and Diagnostic... Mar 2016Pancreatoblastoma, is rare exocrine malignant tumour of childhood. We are reporting a case of three-year-old child presented to our hospital suffering from vague...
Pancreatoblastoma, is rare exocrine malignant tumour of childhood. We are reporting a case of three-year-old child presented to our hospital suffering from vague abdominal pain for further examination and treatment. Clinical examination revealed only a palpable abdominal mass. CT Scan revealed a huge complex space occupying lesion 9.1x8.8x9.2cm with large central cystic degeneration and lobulated enhancing peripheral solid components with foci of calcification, seem to arise from body and tail regions of pancreas. Surgery was done and mass was removed. By histopathology and immunohistochemistry it was diagnosed as pancreatoblastoma. The prognosis is very good in paediatric age, lacking evidence of metastatic disease at first presentation. Therefore early diagnosis is needed for specific treatment. The case is being reported because of its rarity.
PubMed: 27134883
DOI: 10.7860/JCDR/2016/15336.7413 -
International Journal of Organ... 2016Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver...
Organ transplantation in patients with prior malignancy increases the risk of tumor recurrence post-transplantation due to immunosuppression. Only two cases of liver transplantation have so far been reported in children with hepatic metastases from pancreatoblastoma, a rare malignant neoplasm originating from the epithelial exocrine cells of the pancreas. Herein, we describe a case of a successful multi-visceral transplant in a man with intestinal failure after surgical resection of pancreatoblastoma.
PubMed: 27721967
DOI: No ID Found -
The American Journal of Pathology Nov 2001Pancreatoblastomas are unusual malignant neoplasms of the pediatric pancreas that may also rarely affect adults. The molecular pathogenesis of pancreatoblastomas is...
Distinctive molecular genetic alterations in sporadic and familial adenomatous polyposis-associated pancreatoblastomas : frequent alterations in the APC/beta-catenin pathway and chromosome 11p.
Pancreatoblastomas are unusual malignant neoplasms of the pediatric pancreas that may also rarely affect adults. The molecular pathogenesis of pancreatoblastomas is unknown. They are clinicopathologically distinct from adult pancreatic ductal adenocarcinomas, but their occasional occurrence in patients with Beckwith-Wiedemann syndrome and the case presented here of a pancreatoblastoma in an adult patient with familial adenomatous polyposis (FAP) suggests that they might bear a genetic similarity to other infantile embryonal tumors such as hepatoblastomas. We analyzed a series of nine pancreatoblastomas for mutations common to other embryonal malignancies including somatic alterations in the adenomatous polyposis coli (APC)/beta-catenin pathway and chromosome 11p, using immunohistochemistry for beta-catenin, 5q and 11p allelic loss assays, and direct DNA sequencing of exon 3 of the beta-catenin gene and the mutation cluster region of the APC gene. In addition, we analyzed the pancreatoblastomas for alterations found in adult-type pancreatic ductal adenocarcinomas including mutations in the K-ras oncogene and the p53 and DPC4 tumor suppressor genes, using direct DNA sequencing of exon 1 of K-ras and immunohistochemistry for p53 and Dpc4. Allelic loss on chromosome 11p was the most common genetic alteration in pancreatoblastomas, present in 86% (six of seven informative cases). Molecular alterations in the APC/beta-catenin pathway were detected in 67% (six of nine), including five neoplasms with activating mutations of the beta-catenin oncogene and the one FAP-associated tumor with biallelic APC inactivation (germline truncating mutation combined with loss of the wild-type allele); seven neoplasms showed abnormal nuclear accumulation of beta-catenin protein. In contrast, loss of Dpc4 protein expression was present in only two cases (one diffuse and one focal), and no alterations in the K-ras gene or p53 expression were detected. Our findings indicate that pancreatoblastomas are genetically distinct from the more common pancreatic ductal adenocarcinomas, but bear a close molecular pathogenesis to hepatoblastomas. In addition, pancreatoblastoma may represent an extracolonic manifestation of FAP.
Topics: Adenomatous Polyposis Coli; Adenomatous Polyposis Coli Protein; Alleles; Base Sequence; Child; Child, Preschool; Chromosomes, Human, Pair 11; Cytoskeletal Proteins; DNA-Binding Proteins; Female; Genes, p53; Genes, ras; Humans; Loss of Heterozygosity; Male; Middle Aged; Molecular Biology; Mutation; Neoplasms, Germ Cell and Embryonal; Pancreatic Neoplasms; Smad4 Protein; Trans-Activators; beta Catenin
PubMed: 11696422
DOI: 10.1016/s0002-9440(10)63008-8 -
The American Journal of Pathology Apr 2002
Topics: Beckwith-Wiedemann Syndrome; Child, Preschool; Humans; Loss of Heterozygosity; Male; Pancreatic Neoplasms; Proto-Oncogene Proteins; Wnt Proteins; Zebrafish Proteins
PubMed: 11943738
DOI: 10.1016/s0002-9440(10)62580-1 -
Pathology Oncology Research : POR Apr 2012In this report, we describe a classic case of stroma rich neuroblastoma, nodular type in a 22 year old female presented with a pancreatic mass. This rare and unusual...
In this report, we describe a classic case of stroma rich neuroblastoma, nodular type in a 22 year old female presented with a pancreatic mass. This rare and unusual presentation elicits several differential diagnostic categories including solid pseudopapillary tumor, pancreatic endocrine tumor, pancreatoblastoma and PNET. In this report, we tried to differentiate between them depending on the histopathological features and using panel of epithelial and neuroendocrine markers. Although of the rarity of pancreatic neuroblastoma as a primary site of origin, however it should be considered in the differential diagnosis of pancreatic masses in children and young adult. Neuropil and ganglionic differentiation are helpful features to recognize neuroblastoma and differentiate them from other small blue cell tumors. The fatal outcome of adult neuroblastoma confirming the independence of age as a prognostic factor in this neoplasm regardless of stage and histology.
Topics: Adult; Diagnosis, Differential; Fatal Outcome; Female; Humans; Neuroblastoma; Pancreatic Neoplasms
PubMed: 21837482
DOI: 10.1007/s12253-011-9434-3 -
The American Journal of Pathology Mar 2002Acinar cell carcinomas (ACCs) are rare malignant tumors of the exocrine pancreas. The specific molecular alterations that characterize ACCs have not yet been elucidated....
Acinar cell carcinomas (ACCs) are rare malignant tumors of the exocrine pancreas. The specific molecular alterations that characterize ACCs have not yet been elucidated. ACCs are morphologically and genetically distinct from the more common pancreatic ductal adenocarcinomas. Instead, the morphological, immunohistochemical, and clinical features of ACCs overlap with those of another rare pancreatic neoplasm, pancreatoblastoma. We have recently demonstrated a high frequency of allelic loss on chromosome arm 11p and mutations in the APC/beta-catenin pathway in pancreatoblastomas, suggesting that similar alterations might also play a role in the pathogenesis of some ACCs. We analyzed a series of 21 ACCs for somatic alterations in the APC/beta-catenin pathway and for allelic loss on chromosome 11p. In addition, we evaluated the ACCs for alterations in p53 and Dpc4 expression using immunohistochemistry, and for microsatellite instability (MSI) using polymerase chain amplification of a panel of microsatellite markers. Allelic loss on chromosome 11p was the most common genetic alteration in ACCs, present in 50% (6 of 12 informative cases). Molecular alterations in the APC/beta-catenin pathway were detected in 23.5% (4 of 17) of the carcinomas, including one ACC with an activating mutation of the beta-catenin oncogene and three ACCs with truncating APC mutations. One ACC (1 of 13, 7.6%) showed allelic shifts in four of the five markers tested (MSI-high), two (15.4%) showed an allelic shift in only one of the five markers tested (MSI-low), and no shifts were detected in the remaining 10 cases. The MSI-high ACC showed medullary histological features. In contrast, no loss of Dpc4 protein expression or p53 accumulation was detected. These results indicate that ACCs are genetically distinct from pancreatic ductal adenocarcinomas, but some cases contain genetic alterations common to histologically similar pancreatoblastomas.
Topics: Adenomatous Polyposis Coli Protein; Adolescent; Adult; Aged; Carcinoma, Acinar Cell; Child, Preschool; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 5; Cytoskeletal Proteins; Female; Genes, Tumor Suppressor; Humans; Immunohistochemistry; Loss of Heterozygosity; Male; Middle Aged; Mutation; Pancreatic Neoplasms; Signal Transduction; Trans-Activators; beta Catenin
PubMed: 11891193
DOI: 10.1016/s0002-9440(10)64917-6 -
Journal of Indian Association of... 2022Acinar cell carcinoma (ACC) is a rare malignant tumor of the pancreas. A 10-year-old girl presented with a large tumor arising from the pancreatic head. Excision sans...
Acinar cell carcinoma (ACC) is a rare malignant tumor of the pancreas. A 10-year-old girl presented with a large tumor arising from the pancreatic head. Excision sans Whipple's procedure was performed. Histopathology revealed it as ACC. In the context of this case, this rare tumor is being reported to highlight that such tumors arising from the head of the pancreas can be managed successfully without always resorting to a Whipple's procedure.
PubMed: 35733585
DOI: 10.4103/jiaps.JIAPS_29_21 -
The American Journal of Pathology Apr 2002Solid-pseudopapillary tumors (SPTs) are unusual pancreatic neoplasms of low malignant potential that most frequently affect young women. Genetic events contributing to...
Solid-pseudopapillary tumors (SPTs) are unusual pancreatic neoplasms of low malignant potential that most frequently affect young women. Genetic events contributing to the development of SPTs are unknown. Whereas the more common ductal adenocarcinomas of the pancreas essentially never harbor beta-catenin or APC gene mutations, we have recently identified alterations of the APC/beta-catenin pathway in other nonductal pancreatic neoplasms including pancreatoblastomas and acinar cell carcinomas. We analyzed a series of 20 SPTs for somatic alterations of the APC/beta-catenin pathway using immunohistochemistry for beta-catenin protein accumulation, direct DNA sequencing of beta-catenin exon 3, and direct DNA sequencing of the mutation cluster region in exon 15 of the APC gene in those SPTs that did not harbor beta-catenin mutations. Immunohistochemical labeling for cyclin D1 was performed to evaluate the overexpression of this cell-cycle protein as one of the putative downstream effectors of beta-catenin dysregulation. In addition, we analyzed the SPTs for genetic alterations commonly found in pancreatic ductal adenocarcinomas, including mutations in the K-ras oncogene and p53 and DPC4 tumor suppressor genes, using direct DNA sequencing of K-ras and immunostaining for p53 and Dpc4. Almost all SPTs harbored alterations in the APC/beta-catenin pathway. Nuclear accumulation of beta-catenin protein was present in 95% (19 of 20), and activating beta-catenin oncogene mutations were identified in 90% (18 of 20) of the SPTs. Seventy-four percent (14 of 19) showed overexpression of cyclin D1, ranging from 10 to 70% of tumor nuclei. In contrast, no K-ras mutations were present in any of the 20 SPTs, and Dpc4 expression was intact in all 16 SPTs for which immunohistochemical labeling was successful. Overexpression of p53 was limited to only 3 of 19 (15.8%) SPTs. These results emphasize the two distinct, divergent genetic pathways of neoplastic progression in pancreatic ductal and nonductal neoplasms.
Topics: Adolescent; Adult; Aged; Base Sequence; Carcinoma, Ductal, Breast; Cystadenoma, Papillary; Cytoskeletal Proteins; Female; Gene Expression; Genes, p53; Genes, ras; Humans; Male; Middle Aged; Molecular Sequence Data; Mutation; Pancreatic Neoplasms; Trans-Activators; beta Catenin
PubMed: 11943721
DOI: 10.1016/s0002-9440(10)62563-1 -
Srpski Arhiv Za Celokupno Lekarstvo 2004Solid-cystic-pseudopapillary tumor (SCPT) of the pancreas, described by Frantz (1959), is a very rare clinical pathologic entity with relatively low grade malignant...
Solid-cystic-pseudopapillary tumor (SCPT) of the pancreas, described by Frantz (1959), is a very rare clinical pathologic entity with relatively low grade malignant potential. The tumor is more frequent in the body and tail of the pancreas. The majority of patients are young females. About 60% of patients are asymptomatic. Complications such as rupture, bleeding or secondary infections are rare. Metastases of the tumor and local recurrence after surgical treatment are also rare. Prognosis is excellent after complete surgical removal. It is difficult to make a preoperative diagnosis of pancreatic SCPT. The exact diagnosis is based on histological findings. Differential diagnosis should consider pancreatoblastoma, non neoplastic cysts, pancreatic pseudo-cysts and hydatid cyst. This is a case report of 39-year old woman who was admitted to our institution with abdominal discomfort and palpable abdominal mass in the upper abdomen. US and CT scan revealed round neoformation of 60 mm in diameter located in the body of the pancreas. Imaging features were not specific enough to allow for precise diagnosis. Curative R0 left spleno-hemipancreatectomy was performed. Histology of the resected specimen revealed solid and cystic-pseudopapillary tumor of the pancreas. The patient was discharged on postoperative day 7 in a good condition. The patient is well 48 months after the operation, with no impairment of pancreatic endocrine or exocrine function.
Topics: Adult; Diagnosis, Differential; Female; Humans; Pancreatic Neoplasms
PubMed: 15938224
DOI: 10.2298/sarh0412431k -
Archives of Disease in Childhood Dec 1988We describe a boy with recurrent pancreatoblastoma who developed Cushing's syndrome due to inappropriate adrenocorticotrophic hormone secretion.
We describe a boy with recurrent pancreatoblastoma who developed Cushing's syndrome due to inappropriate adrenocorticotrophic hormone secretion.
Topics: Adrenocorticotropic Hormone; Cushing Syndrome; Humans; Infant; Male; Neoplasm Metastasis; Neoplasm Recurrence, Local; Pancreas; Pancreatic Neoplasms
PubMed: 2852926
DOI: 10.1136/adc.63.12.1494